Related Experiment Video
Updated: Jun 10, 2026
07:51
Refined Murine Model of Idiopathic Pulmonary Fibrosis
Published on: June 17, 2025
[Combined pulmonary fibrosis and emphysema]
Karina Portillo Carroz1, Juan Roldán Sánchez, Josep Morera Prat
1Servei de Pneumologia, Hospital Germans Trias i Pujol, Badalona, Barcelona, Spain. karisoe@yahoo.es
Archivos De Bronconeumologia
|August 3, 2010
Summary
The combination of pulmonary fibrosis and emphysema (CPFE) is a lung condition where emphysema and fibrosis coexist. Early recognition of its unique lung function profile is crucial for diagnosis and management.
Area of Science:
- Pulmonology
- Radiology
- Pathophysiology
Context:
- Combination of pulmonary fibrosis and emphysema (CPFE) is a distinct clinical syndrome.
- Characterized by coexisting upper lobe emphysema and lower lobe fibrosis.
- Patients present with seemingly normal lung volumes but reduced carbon monoxide transfer and exercise hypoxemia.
Purpose:
- To define the clinical and functional characteristics of CPFE.
- To highlight the prevalence and prognostic significance of pulmonary hypertension in CPFE.
- To discuss the diagnostic role of high-resolution computed axial tomography (HRCT).
Summary:
- CPFE patients exhibit a unique lung function profile: normal/mildly altered lung volumes with significantly reduced DLco and exercise hypoxemia.
- Pulmonary hypertension is common and a major negative prognostic factor.
- Etiology, particularly the role of cigarette smoking, and pathogenic mechanisms remain unclear, with ongoing research in animal models.
Impact:
- Accurate diagnosis relies on recognizing specific functional and imaging findings.
- Underdiagnosis is likely due to the lack of awareness of its characteristic presentation.
- Further research is needed to clarify pathogenesis and establish therapeutic consensus.
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